Chondrodermatitis nodularis chronica helicis

File:Chondrodermatitis.jpg

Chondrodermatitis helicis nodularis in a 67-year-old man.

1_ Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Karnik Raju Paila Bangaru, M.B.B.S.[2] Kiran Singh, M.D. [3]

Synonyms and keywords:Chondrodermatitis nodularis helicis, winkler disease, ear pressure sore, chondrodermatitis nodularis auricularis

Overview

Chondrodermatitis nodularis chronica helicis (also known as "Chondrodermatitis nodularis helicis") (CNCH) is a small, nodular, tender, chronic inflammatory lesion occurring on the helix of the ear, occurring most often in men.

Historical Perspective[edit | edit source]

• Chondrodermatitis nodularis chronica helicis was first discovered by Max Winkler, a dermatologist, in 1915, in a paper titled Knötchenförmige Erkrankung am Helix .
• in 1925, Foerster further developed the clinical, microscopic and treatment options for CNCH.

Pathophysiology

• The pathophysiology of Chondrodermatitis nodularis chronica helicis remains unclear. It may be due to an inflammatory condition from chronic pressure on helix of the ear, but can occur on anti-helix too.
• CNCH is seen mostly in elderly men. There are few cases seen in women and young, especially when CNCH is associated with autoimmune or connective tissue disorders.
• The anatomy of ear pinna can contribute to the pathophysiology of CNCH. The pinna has limited blood supply and little subcutaneous tissue, which easily lead to formation of sore with pressure or trauma or cold temperature.
• In 2009, Perichondrium vasculitis theory explained above anatomical features. Because of the vasculature and subcutaneous tissue padding limitations, there is easy development of ischemia and necrosis of underlying cartilage, causing a severe inflammatory condition, leading to CNCH.
• There could be a possibility of genetic relation to disease, as observed in a case of monozygotic twins, but not studied enough to make a point.
• On gross pathology, CNCH looks like any other sore. Microscopically, it shows hyperkeratosis, parakeratosis with epithelial hyperplasia, dermal tissue damage with underlying cartilage destruction and dermal vessel proliferation.

Causes[edit | edit source]

• Chondrodermatitis nodularis chronica helicis may be caused due to chronic pressure or trauma or anatomic features or temperature changes or actinic damage to the ear. Prolonged pressure seemed the most commonly reported cause. Sleeping on the one side can cause this pressure.

• Other causes can be continuous use of headphones/ ear phones/ cell phones, hearing aids, or can be idiopathic. There are some theories on association with autoimmune disorders and connective tissue diseases like scleroderma, autoimmune thyroiditis, lupus erythematosus, or dermatomyositis.

Differentiating [disease name] from other Diseases[edit | edit source]

• Chondrodermatitis nodularis chronica helicis can present as painful nodular lesion with central crust, so it must be differentiated from Basal cell carcinoma which is generally painless and with more vascularity.
• It must be differentiated from other diseases that cause a bump on the ear, such as:

• Actinic Keratosis
• Keratoacanthoma
• Squamous cell carcinoma
• Gouty tophi

Epidemiology and Demographics[edit | edit source]

• The disease is much common than it is reported.

Age[edit | edit source]

• CNCH is more commonly observed among patients aged 50-80 years old, but has been seen in young adults and children too. Unilateral occurrence is more common.

Gender[edit | edit source]

• CNCH is seen mostly in elderly men, but may occur with women and children too.

Race[edit | edit source]

• There is no racial predilection for CNCH, but is seen more commonly in fair skinned with sun exposure.

Risk Factors[edit | edit source]

• Common risk factors in the development of CNCH are pressure, trauma, sun damage, and systemic conditions.

Natural History, Complications and Prognosis[edit | edit source]

• Early clinical features include just a painful sore. Spontaneous resolution is rare.
• Prognosis is generally very good with treatment, although long term morbidity is common. Remissions can be seen when undertreated.

Diagnosis

History and Symptoms[edit | edit source]

• CNCH mostly presents as a spontaneously appearing round to oval tender nodule with raised edges on helix or anti-helix with a central crust/ulcer, commonly on ear on usually sleeping side, with nocturnal pain.
• Like said, this is most common presentation, but it can also present as bilateral lesion, or with diurnal pain.
• It usually grows and becomes stable after reaching it's maximum size.

Physical Examination

Ear

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  Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.

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  Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.

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  Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.

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  Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.

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  Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.

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  Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.

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  Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.

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  Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.

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  Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.

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  Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.

Laboratory Findings[edit | edit source]

• There are no specific laboratory findings associated with CNCH.

• Skin biopsy may be necessary in conditions to differentiate it from carcinomatous lesions.

Other Diagnostic Studies[edit | edit source]

• Investigating for the systemic illness( autoimmune diseases, connective tissue diseases or chronic non-communicable diseases) for underlying associations to the disease is indicated in some studies.

Treatment[edit | edit source]

Medical Therapy[edit | edit source]

• There is no treatment for [disease name]; the mainstay of therapy is supportive care.

• The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action 1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery[edit | edit source]

• Surgery is the mainstay of therapy for
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention[edit | edit source]

• Effective measures for the primary prevention of CNCH include avoiding ear pressure/ pressure offloading techniques.

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

References

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